Diagnostic Potential of 3D-Data–Based Reconstruction Software: An Analysis of the Rare Disease Pattern of Cherubism

Abstract

Cherubism is an autosomal-dominant syndrome characterized by bilateral maxillomandibular bony degeneration, fibrous connective tissue hyperplasia, and displacement of permanent tooth germs. Reossification of the cystic lumen occurs spontaneously, but dislocated teeth must be realigned orthodontically. Advancements in virtual 3D reconstruction of anatomic structures based on computed tomography (CT) or cone beam CT data have provided for more predictable individual treatment planning. We evaluated two software programs for making densitometry and volume measurements of cystic areas in the mandibles, and for 3D visualization of permanent tooth germs within the cystic lumen, in two siblings with cherubism.